Scleromyxedema:Satisfying Response to Etretinate -report of one case-

鞏皮性黏液水腫：對Etretinate有療效 –病例報告–

鍾政瑾(Chen-Chin Chung) ； 謝旭榮(Hsu-Jung Hsieh) ； 林智一(Tze-Yi Lin)

《中華皮膚科醫學雜誌》 18卷1期 (2000/03) Pp. 65-73

摘要

一位28歲男性，一年來因在前額、背部、胸部、肩膀、手肘、手背及下腿長了許多膚色到紅色的結節；其前額、頭皮、耳後、頸背、腋下也長有許多大小約略相同呈線狀分佈之丘疹，部份並融合成硬化苔蘚樣的班塊，而來本院求診。我們各在其頸背及手肘取苔蘚樣的班塊及結節做組織病理學的檢查，其顯示上真皮層有黏液沉積，其特殊染色包括alcian blue at pH 2.5及colloidal iron stain均顯示陽性，表示有acid mucopolysacchride沉積。依據其臨床及病理表現，我們將其診斷為黏液水腫性硬皮症。病人經口服etretinate(Tigason) 1mg/kg/day，５週後，其病灶有明顯的軟化及縮小。藥量因此隆至0.5mg/kg/day至今約有8個月，病況有顯著的改善。

並列摘要

A 28-year-old male patient presented with multiple flesh-colored to erythematous nodules over the forehead, back, chest, scapula, elbows, both hands and lower legs, and numerous small uniform-sized papules in linear pattern on indurated skin over scalp, retroauricular area, nape of the neck, and axillae for about one year. Histopathology of the dermal nodule over the elbow and the plaque over the nape of the neck showed mucin deposition in upper dermis which was positive with alcian blue at pH 2.5 and colloidal iron stain. Diagnosis of scleromyxedema was made. He was treated with etretinate (Tigasone) 1mg/kg/day. Softening and decrease in size of dermal nodules were noted at the 5th week. The dose was then reduced to 0.5mg/kg/day and maintained for about 8months. Marked improvement was observed.

並列關鍵字

Scleromyxedema ； Etretinate